The Chiari I malformation (CM-I) is due to congenital hypoplasia of the posterior fossa and crowding at the craniocervical junction resulting in variable obstruction of spinal fluid flow from the cranial to the spinal compartments. This obstruction may, or may not, cause the development of a cavity (syrinx) within the spinal cord.
An unknown percentage of patients with CM-I will develop neurological symptoms, most often in adult life. The symptoms vary widely and are related to spinal fluid obstruction, tissue compression, repetitive impaction of neurological tissue, and/or syringomyelia.
MRI and CINE phase-contrast MRI are the diagnostic tests of choice.
Persons with characteristic symptoms not due to other causes, with supportive findings on the MRI studies, and with progressive neurological findings or refractory symptoms impacting the quality of life are candidates for surgery.
Many people, though not all, respond favorably to surgical treatment - most often posterior fossa decompression. A variety of procedures are used, but no consensus exists. Future advances in basic research, diagnostic technologies, and medical and surgical treatments should improve the outlook for those burdened with this condition and lead to a cure.
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This blog will explore many of the details of the Chiari I malformation noted above and the Chiari II, and III malformations. Associated disorders including syringomyelia and tethered cord will also be covered. It will search for weakness and errors in our understanding, and, hopefully, increase awareness of these disorders to the benefit of those affected.
Posted byJohn Oro', MD
The Chiari I malformation (CM-I) is due to congenital hypoplasia of the posterior fossa and crowding at the craniocervical junction resulting in variable obstruction of spinal fluid flow from the cranial to the spinal compartments. This obstruction may, or may not, cause the development of a cavity (syrinx) within the spinal cord.
An unknown percentage of patients with CM-I will develop neurological symptoms, most often in adult life. The symptoms vary widely and are related to spinal fluid obstruction, tissue compression, repetitive impaction of neurological tissue, and/or syringomyelia.
MRI and CINE phase-contrast MRI are the diagnostic tests of choice.
Persons with characteristic symptoms not due to other causes, with supportive findings on the MRI studies, and with progressive neurological findings or refractory symptoms impacting the quality of life are candidates for surgery.
Many people, though not all, respond favorably to surgical treatment - most often posterior fossa decompression. A variety of procedures are used, but no consensus exists. Future advances in basic research, diagnostic technologies, and medical and surgical treatments should improve the outlook for those burdened with this condition and lead to a cure.
__________________________________________
This blog will explore many of the details of the Chiari I malformation noted above and the Chiari II, and III malformations. Associated disorders including syringomyelia and tethered cord will also be covered. It will search for weakness and errors in our understanding, and, hopefully, increase awareness of these disorders to the benefit of those affected.
Posted byJohn Oro', MD